Epstein–Barr virus associated lymphoproliferative disorder (EBV-LPD) occurs in patients with immunodeficiency, but it has not been well described in patients who have received chemotherapy for solid tumors. We describe a child with rhabdomyosarcoma who developed isolated central nervous system (CNS) EBV-LPD during combination chemotherapy with vincristine, actinomycin D and cyclophosphamide. The patient was treated with high-dose methotrexate (HD-MTX) for CNS EBV-LPD and then treated with rituximab in addition to HD-MTX because of the emergence of LPD in the liver. I.v. rituximab combined with HD-MTX might be effective therapy for CNS EBV-LPD.